Primary congenital abdominal aortic aneurysm associated with renovascular hypertension in pediatric patient.

Idiopathic congenital abdominal aortic aneurysm (AAA) is rare, with only a few cases reported in the literature. We present a case of a 3-year-old girl with a fusiform AAA measuring 19 mm in segments 4 and 5, associated with renal artery stenosis and severe hypertension. She was treated with three antihypertensive medications. Initially, left nephrectomy was performed due to the persistence of hypertension. Subsequently, we conducted a hepatorenal bypass and open surgical repair of the AAA via left thoracolumbar approach. At the 4-year follow-up, there were no complications. Pediatric CAAA is very rare. Case-by-case discussion and management are essential; the open approach with synthetic grafts is the gold standard and has resulted in good outcomes.